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PERSPECTIVES: Because therapeutic options for adult patients with advanced or metastatic soft tissue sarcoma are scarce and the possibility of cure for these patients is extremely limited, the Sarcoma Disease Site Group (dsg) felt that a review of the available literature on dose-intensive chemotherapy for adult patients with locally advanced or metastatic soft tissue sarcoma and subsequent development of a clinical practice guideline based on the evidence were important.

The practice guideline report was reviewed and approved by the Sarcoma dsg, which comprises medical oncologists, radiation oncologists, surgeons, a pathologist, a methodologist, and community representatives.

PRACTICE GUIDELINE: Based on the systematic review, consensus, and external review, the Sarcoma dsg makes these recommendations: Dose-intensive chemotherapy with growth factor support is not recommended in the first-line treatment of patients with inoperable locally advanced or metastatic soft tissue sarcoma.

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On the basis of the available evidence, high-dose chemotherapy with growth factor or autologous bone marrow/stem cell transplantation should not be used in the routine treatment of patients with inoperable, locally advanced, or metastatic soft tissue sarcoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Chemotherapy in adult soft tissue sarcoma.

Recent results of various meta-analyses and development of newer drugs have changed the medical management of soft tissue sarcoma.

We have carried out an extensive search in PubMed, Medline for almost all relevant articles concerning chemotherapy of soft tissue sarcoma.

In locally advanced and metastatic STSs, single agent adriamycin remains the basic standard of medication.

Newer methodologies such as, Bayesian adaptive randomization and inclusion of newer end points like progression-free rate, time of progression rate, and tumor growth rate will improve the results of sarcoma trials.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Small round cell sarcoma, such as Ewing/PNET, desmoplastic small round cell sarcoma and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies, which are treated with multimodality, dose-intensitive and neoadjuvant protocols regardless of size or overt metastatic disease.

A limited number of effective agents available for the treatment of patients with metastatic adult soft-tissue sarcoma exists, which have failed anthracyline and ifosfamide-based chemotherapy.

Most other high-grade (grading >I) so-called adult-type soft-tissue sarcomas such as fibro, lipo, pleomorphic and synovial sarcoma are treated with a anthracycline-based regimen with or without ifosfamide as front-line therapy.

In this review, the therapeutic activities of drugs currently available as second-line treatment in patients with metastatic soft tissue sarcoma are summarized, providing an overview of contentious or emerging treatment issues.

In relapsed 'adult-type' soft-tissue sarcomas trofosfamide, gemcitabine and ecteinascidin (ET-743) appear to be drugs associated with moderate activity and an acceptable toxicity profile.

An interesting finding to be noted is that the different drugs have particular effects in distinct subtypes of soft-tissue sarcoma; however, it has to be taken into account that the number of patients included in those phase II trials are limited.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The number of effective cytotoxic agents for the treatment of patients with metastatic adult soft tissue sarcoma is limited, especially when patients have failed anthracycline- and ifosfamide-based chemotherapy.

Small round cell tumours (SRCTs), such as Ewing's sarcoma/primitive neuroectodermal tumour, desmoplastic SRCT and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies, which are treated with multimodality, dose-intensive, neoadjuvant protocols regardless of size or overt metastatic disease.

Most other high-grade (grading >I), so-called 'adult type', soft tissue sarcomas such as fibrosarcoma, liposarcoma, pleomorphic and synovial sarcomas are treated with an anthracycline-based regimen with or without ifosfamide as front-line therapy.

In relapsed 'adult type' soft tissue sarcomas, trofosfamide, gemcitabine and trabectedin (ecteinascidin 743) appear to be drugs associated with some activity and an acceptable toxicity profile.

A high activity has been reported for the taxanes, in particular for paclitaxel, in vascular sarcomas located in the scalp or face and in Kaposi's sarcoma.

It is interesting to note that the different drugs have particular effects in distinct subtypes of soft tissue sarcoma; however, it should be taken into account that the number of patients included in the phase II trials is limited.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Using single-agent therapy in adult patients with advanced soft tissue sarcoma can still be considered standard care.

The group of soft tissue sarcomas in adult patients is a heterogeneous group with more than 40 different subtypes.

While local treatment remains the mainstay for localized disease, systemic chemotherapy can importantly contribute in the treatment of advanced soft tissue sarcoma.

For patients with metastatic disease, chemotherapy is a palliative treatment in the vast majority of the cases.

This review addresses the current available data on chemotherapy for adult patients with soft tissue sarcoma, excluding gastrointestinal stromal tumor, the Ewing-like sarcomas, and other small blue round cell tumors.

In addition, it is increasingly recognized that future research in soft tissue sarcoma should focus on the identification of tumor factors that can serve as targets for treatment and that the diverse tumor subtypes should be analyzed separately for their sensitivity to systemic treatment.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND: The benefit of chemotherapy in the treatment of primary soft-tissue sarcoma (STS) is controversial.

To the authors' knowledge, few studies to date have examined the effect of chemotherapy in patients undergoing pulmonary resection for metastatic STS of the extremity.

CONCLUSIONS: Although it is difficult to completely control for the effects of selection bias on outcome in this highly selected cohort of patients, data from the current study suggest that systemic chemotherapy has minimal, if any, long-term impact on the outcome of patients undergoing pulmonary resection for metastatic STS of the extremity.

In the last decade, locally advanced and metastatic soft tissue sarcoma have been managed only through surgery, radiotherapy and standard chemotherapy (mainly based on anthracycline and ifosfamide).

AREAS COVERED IN THIS REVIEW: In the present article, all the main new molecules under clinical evaluation for the treatment of soft tissue sarcoma are revised by describing the mechanism of action, the biological rationale of their use in sarcoma and by reporting the available data about safety and efficacy, up to 2009.

WHAT THE READER WILL GAIN: A brief summary of the standard treatments available at the moment and a complete analysis of the state of art about the development of new target therapies in the management of soft tissue sarcoma.

TAKE HOME MESSAGE: The identification of new biological therapies that target soft tissue sarcoma tumorigenesis key points seems to offer a real opportunity of improving the prognosis of this often aggressive disease.

In this sense, the best management for soft tissue sarcoma patients is in a clinical trial and participation in clinical trials should be encouraged.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Small round cell sarcomas (SRCT), such as Ewing/primitive neuroectodermal tumor, desmoplastic SRCT, and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies that are treated with multimodality dose-intensive neoadjuvant protocols regardless of size or overt metastatic disease.

However, the number of effective cytotoxic agents for the treatment of patients with metastatic so-called adult soft tissue sarcoma is limited, especially when patients have failed anthracycline- and ifosfamide-based chemotherapy.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND AND AIMS: Synchronous and heterochronous multiple soft tissue sarcoma of the extremities is very rare.

Out of 1,201 of our patients, 4 patients presented with symmetrical bilateral soft tissue sarcoma of the extremities.

RESULTS: All tumours were located at the extremities and were diagnosed as leiomyosarcoma in two patients, malignant fibrous histiocytoma and clear cell sarcoma in one patient each.

In two patients a second primary sarcoma of the same entity was considered the most likely diagnosis, whereas in one patient a contralateral lymph node metastasis and in one other patient an atypical soft tissue metastasis had to be taken into account.

Aside from irradiation effects, exposition to other carcinogenic agents or genetic predisposition, the reasons for the clustering of soft tissue sarcoma in one same patient remain still unclear.

Only one patient, although suffering from disseminated metastatic disease was living at follow-up time, the other three patients had already died.

CONCLUSION: The interpretation of the bilateral manifestation of soft tissue sarcoma remains open, but predicts an unfavourable outcome.

BACKGROUND: Soft tissue sarcomas (STS) are uncommon malignancies and elderly STS patients have been reported to receive less definitive treatment compared to young STS patients.

The reasons for no treatment were irresectability of the sarcoma (65%), metastatic disease (11%), comorbidity (4%), poor general health (5%), death prior to therapy (7%) and refusal of therapy (3%) (motivation not documented in 5%).

Withholding treatment was mostly disease-related (76%), e.g. irresectable retroperitoneal STS or metastatic disease; for 19% of the patients, it was related to their poor general health.

Until only a few years ago, treatment choices for metastatic STS were easy to review because of the very limited number of active compounds available.

In order to be successful, it may be necessary to combine not only different compounds but also different targets beyond the proliferation machinery of sarcoma cells such as tumor angiogenesis, the tumor stromal compartment, or tumor cell oncogene products.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Enhanced expression levels of NG2 proteoglycan in presurgical original lesions of soft-tissue sarcoma (STS) patients defines with 55% probability the immediate (i.e., within 12 months postsurgery) risk in these individuals to develop postsurgical secondary lesions, independently of any other clinical trait.

Evaluation of the timing of metastasis formation in patients with high and low levels of NG2 in their primitive lesions further stratified the patients in subsets with diverse lag phases in the occurrence of metastatic disease.

In our cohort of high-grade STS cases, transcription of NG2 also showed a 81-fold amplification in metastatic lesions, when compared to primitive ones, and this gene overexpression was accompanied by an abundant but nonuniform in situ expression of its product.

In a similar manner as seen in primitive lesions, patients with higher levels of metastatic NG2 encountered a significantly more dismal clinical course.

Therefore, minimally invasive assessment of the transcription levels of the NG2 gene represents a parameter capable of predicting the arising of metastatic disease within a definite postsurgery time interval, and affords in adjunct in the definition of life expectance in STS patients.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

PURPOSE OF REVIEW: We give an overview on the emerging compounds for patients with soft tissue sarcoma.

RECENT FINDINGS: Soft tissue sarcomas are a heterogeneous group of tumours that arise predominantly from the embryonic mesoderm.

They account for fewer than 1% of all adult malignancies.

The prognosis of patients with advanced metastatic soft tissue sarcoma remains poor, with disease-free survival at 5 years below 10%.

SUMMARY: Understanding of the molecular biology and pathogenesis of soft tissue sarcomas has been enhanced, and in the near future this should translate into molecular tumour characterization and development of new therapeutic strategies.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

AIM: Radiotherapy has been shown to improve local control in combination with limb-sparing or conservative surgery in the management of localised soft tissue sarcoma.

METHODS: Consecutive patients with localised extremity or truncal soft tissue sarcoma who presented between January 1996 and December 2000 and treated with preoperative radiotherapy followed by limb-sparing surgery were reviewed.

Patients with recurrent disease or metastatic disease at diagnosis and patients below the age of 16years were excluded.

CONCLUSION: Preoperative radiotherapy followed by surgery provides effective local control in the management of soft tissue sarcoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Metastatic monophasic synovial sarcoma of the pleura.

Pleural metastasis of synovial sarcoma that originally developed in the soft tissue is a very rare entity.

An asymptomatic 25-year-old female, with a history of a resected synovial sarcoma in her left brachial muscle and pulmonary metastasectomy of the right lung, presented a small nodule in the periphery of the left lung on a routine chest-computed tomography.

A soft flat red tumor approximately 2 cm in diameter was shown on the pleura of the lingula, mimicking a blood clot on the pleura.

We diagnosed unresectable pleural metastases of synovial sarcoma and finished the operation after sampling another pulmonary pleural lesion.

Postoperative histopathological examination revealed a solid and bundle-like proliferation of a short spindle cell tumor with a monophasic pattern, which was diagnosed as a metastatic pleural synovial sarcoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

INTRODUCTION: As adult urological soft tissue sarcomas (STS) are rare, there have been few recent large-scale studies of these tumors.

This report describes a single institutional experience of adult urological STS over 25 years.

PATIENTS AND METHODS: The study population consisted of 25 adult patients with histologically diagnosed STS arising in the urinary tract, male genital system, or retroperitoneum between January 1983 and July 2008.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

However, for patients with locally advanced or metastatic disease chemotherapeutic treatments have not greatly changed the poor outcome of the disease.

For patients with metastatic sarcoma long-term survival can only be achieved in a small number of patients with mostly resectable disease.

A variety of new drugs or drug combinations seem to exhibit considerable activity in certain histological sarcoma subtypes, which may soon broaden the armamentarium of drugs for a subset of patients.

However, with the vastly improved understanding of the biology and pathology of soft tissue sarcoma an era of opportunities seems to have begun and the recent success in the treatment of gastrointestinal stromal tumors impressively shows how fast a gain in the understanding of oncogenic mechanisms may translate into a highly efficient, clinically useful treatment.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The IVADo regimen--a pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma: a pilot study of behalf of the European pediatric Soft tissue sarcoma Study Group.

BACKGROUND: The role of doxorubicin (Doxo) as part of multidrug regimens used to treat children with soft tissue sarcoma (STS) is controversial.

To evaluate the feasibility of combining Doxo with the well established ifosfamide, vincristine, and actinomycin D (IVA) regimen, the Italian STS Committee performed a pilot study on a series of children with metastatic STS.

This combination will be investigated in high-risk patients with rhabdomyosarcoma in a randomized trial launched by the European pediatric Soft tissue sarcoma Study Group.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] New drugs for the treatment of metastatic or refractory soft tissue sarcomas in children.

Children with relapsed, recurrent or metastatic sarcomas represent a therapeutic challenge for the pediatric oncologist.

For example, drug development in rhabdomyosarcoma has relied upon the preclinical xenograft model, whereas therapies for pediatric nonrhabdomyosarcomatous soft tissue sarcomas have mostly been derived from adult trials.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Pulmonary nodules discovered during the initial evaluation of pediatric patients with bone and soft-tissue sarcoma.

BACKGROUND: Recent technical advances in CT imaging and data processing have improved the ability to detect small pulmonary nodules in children with bone and soft-tissue sarcoma undergoing radiologic imaging of the chest.

PROCEDURE: We retrospectively studied medical records and CT chest scans at initial diagnosis of 210 children and young adults presenting to a single pediatric tertiary care hospital specialized in oncology for evaluation of bone or soft-tissue sarcoma.

Of patients who underwent biopsy or resection of a nodule, 41.7% had metastatic pulmonary disease.

Having more than three nodules and a bilateral distribution were associated with histology-proven metastasis (P = 0.002 and P = 0.011, respectively) and an increased frequency of recurrent or progressive metastatic disease in the lung (P < 0.001 and P = 0.023, respectively).

CONCLUSIONS: Pulmonary nodules were commonly detected during the initial evaluation of pediatric cases of sarcoma.

Number and distribution of nodules were significantly associated with metastatic disease and outcome.

PURPOSE: Gemcitabine as a single agent and the combination of gemcitabine and docetaxel have activity in patients with metastatic soft tissue sarcoma.

To determine if the addition of docetaxel to gemcitabine improved clinical outcome of patients with metastatic soft tissue sarcomas, we compared a fixed dose rate infusion of gemcitabine versus a lower dose of gemcitabine with docetaxel.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

PATIENTS: Between January 1, 1991, and December 31, 2002, 61 patients (33 men and 28 women; median age at initial diagnosis, 42 years [age range, 18-74 years]) were surgically treated for pulmonary metastases of soft tissue sarcoma at University Hospital, Hamburg-Eppendorf, Germany.

The mean number of resected pulmonary metastatic lesions was 5 (range, 1-48).

The number of resected lung metastatic lesions had no prognostic relevance (P = .37).

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Phase II trial of gemcitabine as first line chemotherapy in patients with metastatic or unresectable soft tissue sarcoma.

OBJECTIVE: The availability of effective chemotherapy agents or regimens for soft tissue sarcomas (STS) is limited.

A multi-institutional study of gemcitabine in patients with STS, without prior chemotherapy for metastatic disease, was initiated in the Southwest Oncology Group May 1, 1998 and completed March 15, 1999.

MATERIALS AND METHODS: Patients were required to have metastatic or unresectable STS with no prior chemotherapy for metastatic disease.

[Title] Standardized approach to the treatment of adult soft tissue sarcoma of the extremities.

Soft tissue sarcomas are very rare tumors.

Several prognostic factors have been identified, including grading, tumor size and development of metastatic disease; however, the relevance of other important aspects in the treatment of patients with soft tissue sarcomas remains unknown or subject to controversy.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Standards and novel therapeutic options in the treatment of patients with soft tissue sarcoma.

Soft tissue sarcomas are a heterogeneous group of tumours arising predominantly from the embryonic mesoderm.

They account for less than 1% of all adult malignancies.

The prognosis of patients with advanced metastatic soft tissue sarcoma remains poor with a disease-free survival at 5 years less than 10%.

Complete resection remains the major factor in providing cure with limited benefits in local tumour control by radiotherapy and only minimal benefit of systemic therapy for metastatic disease.

Moreover, we give an overview on the emerging compounds for patients with soft tissue sarcoma including recent developments of targeted therapy focusing on antiangiogenic and immunomodulatory drugs, Bcl-2 antisense therapy, raf kinase and mTOR inhibition, minor groove binders, and other agents being developed.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Phase III trial of two investigational schedules of ifosfamide compared with standard-dose doxorubicin in advanced or metastatic soft tissue sarcoma: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Hepatic metastasectomy for soft-tissue sarcomas: is it justified?

BACKGROUND: Except for patients with gastrointestinal stromal tumors (GIST), systemic chemotherapy in patients with liver metastasis of soft-tissue sarcoma (STS) is not effective.

RESULTS: Twenty-seven of 45 patients with liver metastasis underwent hepatic resection; 59% of these patients had a solitary metastasis, 22% had two metastases, and 18% had three or more metastatic nodules.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND: The number of effective cytotoxic agents for the treatment of patients with metastatic adult type soft tissue sarcoma (STS) is limited, when patients have failed anthracyline-based chemotherapy.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Clear cell sarcoma of soft parts is a rare soft tissue malignancy that shows phenotypic overlap with cutaneous melanoma but can be distinguished by the presence of a t(12;22) translocation.

Microsatellite instability (MSI), a variation in the lengths of short repeat DNA segments in the genome, has been implicated in melanoma tumorigenesis, but is rare or absent in clear cell sarcoma.

To determine if the association between MMR defects and MSI extends to clear cell sarcoma, we compared a group of nine clear cell sarcomas to 11 metastatic melanomas on the basis of MSI and the expression of MMR proteins.

Immunohistochemistry was evaluated on formalin-fixed paraffin-embedded tissue for hMLH1, hMHS2 and hMSH6.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Purpose. Patients with soft tissue sarcomas (STS) are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult.

In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting development of second primary STS.

Discussion. The similarities and dissimilarities identified in the first and second STS suggest that genetic profiles can be used to distinguish soft tissue metastases from second primary STS.

The demonstration of genetically different soft tissue sarcomas in the same patient suggests independent tumor origin and serves as a reminder to consider development of second primary STS, which has prognostic and therapeutic implications.

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[Publication-country] United States

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Outcomes of re-excision after unplanned excisions of soft-tissue sarcomas.

BACKGROUND AND OBJECTIVES: Unplanned excisions of soft-tissue sarcomas of the extremities occur commonly.

Our goal was to evaluate the presence of residual disease, the treatment outcomes as they relate to local and distant recurrence and 5-year survival, and the limb functional outcomes in patients with unplanned sarcoma excision who were treated with re-excision and adjuvant therapy.

Of those 42 patients, 38 without gross residual disease or metastatic lesions formed the basis of this review.

Univariate analysis showed that stage-III disease (American Joint Committee on Cancer classification of soft-tissue sarcomas), lesions below the fascia, a histologic high-grade, and the development of organ metastasis were statistically significant factors for mortality.

Stage-III disease also was significant for mortality on multivariate analysis.

Only stage-III disease was significant for the development of local recurrence.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Brostallicin, an agent with potential activity in metastatic soft tissue sarcoma: a phase II study from the European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.

The study aimed to assess the efficacy and safety of brostallicin, a new DNA minor groove binder, at a dose of 10mg/m2, intravenous (i.v.) every three weeks, in patients with advanced or inoperable soft tissue sarcoma (STS) and gastrointestinal stromal tumour (GIST) failing first line therapy.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND: Nowadays, the results of the management of malignant bone and soft-tissue tumors have been dramatically improved because of the advance in imaging, chemotherapy, radiation therapy, and surgical techniques.

From March 1994 to July 2006, 32 endoprosthetic reconstructions were performed on 30 patients following malignant bone or soft-tissue tumor removal.

The diagnosis was conventional osteosarcoma in 16 patients, parosteal osteosarcoma in two patients, chondrosarcoma in two patients, leiomyosarcoma in two patients, failed allograft in two patients and one patient each of periosteal osteosarcoma, Ewing's sarcoma, Gorham's disease, synovial sarcoma, malignant fibrous histiocytoma, metastatic renal cell carcinoma, and prosthetic loosening.

CONCLUSION: Endoprosthetic reconstruction could yield satisfactory results as a wide excision and limb-salvage for patients with malignant bone and soft-tissue tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Is there an indication for high-dose chemotherapy in the treatment of bone and soft-tissue sarcoma?

Sarcomas represent a rare and heterogeneous disease, and the prognosis of patients with unresectable or advanced metastatic bone and especially soft-tissue sarcomas remains poor, with a disease-free survival of less than 10% at 5 years.

However, all studies comprised small patient cohorts and included very heterogeneous histological subtypes of soft-tissue sarcoma.

In this review, we provide an overview of the literature concerning high-dose chemotherapy with hematopoietic stem cell support in the treatment of bone and soft-tissue sarcomas.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Phase I study of non-pegylated liposomal doxorubicin in combination with ifosfamide in adult patients with metastatic soft tissue sarcomas.

OBJECTIVE: The aim of this study was to evaluate the maximum tolerated dose (MTD) and safety of the combination of non- pegylated liposomal doxorubicin (Myocet) and ifosfamide in patients with metastatic soft tissue sarcomas.

METHODS: Cohorts of four patients with metastatic soft tissue sarcomas received up to five cycles of intravenous ifosfamide 3000 mg/m2 on days 1- 3 in combination with escalating doses of intravenous Myocet on day 1 every 3 weeks until dose limiting toxicity (DLT) in at least one patient.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Extremity soft tissue sarcomas presented as hematomas.

INTRODUCTION: Soft tissue sarcoma (STS) with extensive intra-tumoral hemorrhage is an infrequently described entity, usually misdiagnosed as intra-muscular hematoma.

MATERIALS AND METHODS: We retrospectively identified 15 patients, with initial clinical or imaging diagnosis of hematoma, or hematoma versus hemorrhagic sarcoma, although final diagnosis of high-grade STS was established in all cases.

Ten patients were referred for further evaluation with the initial diagnosis of muscle strain/hematoma, hematoma versus abscess in one, whereas four were referred for soft tissue mass evaluation.

One patient had lung metastatic disease at presentation and eight developed lung metastases within a median time of 7 months.

The evacuation of hematomas should include pathologic examination of tissue.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

QUESTIONS: In adult patients with inoperable locally advanced or metastatic soft-tissue sarcoma, do combination chemotherapy regimens containing ifosfamide have an advantage in terms of response rate, time to progression, or survival, as compared with similar regimens without ifosfamide when used as first-line therapy?

A number of studies have suggested a schedule and a dose-response relationship for ifosfamide in metastatic sts.

Given the limited effective therapeutic options available for patients with metastatic sts, the Sarcoma Disease Site Group (dsg) felt that a need existed to more specifically evaluate the potential benefits of ifosfamide-containing combination chemotherapy in that setting.

The Sarcoma dsg developed an evidence-based series report through systematic review, evidence synthesis, and input from practitioners across Ontario.

The report was reviewed and approved by the Sarcoma dsg, which comprises medical oncologists, radiation oncologists, surgeons, methodologists, and patient representatives.

PRACTICE GUIDELINE: In patients with metastatic sts, the addition of ifosfamide to standard first-line doxorubicin-containing regimens is not recommended over single-agent doxorubicin.

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[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Surgical management of metastatic sarcoma to the brain.

OBJECT: Metastatic sarcoma to the brain is rare and represents a therapeutic challenge due to its relative resistance to radio- and chemotherapy.

The authors reviewed a series of patients with metastatic sarcoma to the brain treated surgically to determine outcomes and identify predictors of survival in these patients.

METHODS: A retrospective review of prospectively collected data was undertaken on patients undergoing surgery between 1993 and 2005 for metastatic sarcoma to the brain at The University of Texas, M.D.

RESULTS: During the study period, 62 patients underwent 84 operations for metastatic sarcoma to the brain.

In multivariate and univariate analysis, control of systemic disease, and sarcomas originating from bone, cartilage, or soft tissue were predictors of survival.

In patients with alveolar soft-part sarcoma, there was a significantly increased survival advantage compared with all other histological subgroups.

CONCLUSIONS: The authors' results suggest that in selected patients, resection of metastatic sarcoma to the brain is associated with a relatively low risk of operative death and results in improvement in neurological function.

Patients with systemic control of their primary disease and certain histological subtypes (specifically alveolar soft-part sarcoma) have improved overall and progression-free survival.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] High incidence of metastatic disease in primary high grade and large extremity soft tissue sarcomas treated without chemotherapy.

BACKGROUND: The risk of metastasis and the survival in patients with primary extremity soft tissue sarcomas is worse when tumour size is large and the grade of malignancy is high.

The question arises if the kind of RTX in the absence of chemotherapy influences the outcome concerning local control, metastatic disease, survival and complications.

METHODS: We retrospectively reviewed the clinical outcome of 233 patients with a primary extremity soft tissue sarcoma treated between 1990 - 2000 with a mean follow-up of 35.8 (4-120) months in our institute.

41 patients had high grade, deep and large tumours (>8 cm), an AJCC stage III (no evidence of metastasis prior to treatment) and were treated with limb salvage surgery and irradiation but stayed without additional chemotherapy.

RESULTS: 56% (23/41) of the population developed metastatic disease, 24% (10/41) local recurrence.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

PURPOSE: The purpose of this trial was to assess the objective clinical response, toxicity, and time to progression of treatment with 9-Nitro-Camptothecin (9-NC) in patients with advanced chordoma, soft tissue sarcoma (STS), and gastrointestinal stromal tumor (GIST).

PATIENTS AND METHODS: Patients with locally advanced and/or metastatic chordoma, STS, or GIST received 9-NC 1.25 mg/m2 orally for 5 consecutive days followed by 2 days of rest.

CONCLUSION: 9-NC has modest activity in delaying progression in patients with unresectable or metastatic chordoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] The pharmacologic basis of ifosfamide use in adult patients with advanced soft tissue sarcomas.

The treatment outcome of patients with locally advanced and metastatic soft tissue sarcomas is poor.

This review addresses the clinical pharmacology, metabolism, and present role of ifosfamide in the treatment of locally advanced and/or metastatic soft tissue sarcomas, excluding gastrointestinal stromal tumors, the Ewing-like sarcomas, and other small blue round cell tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The therapeutic approach to soft tissue sarcomas (STS) has evolved over the past two decades based on the results from randomized controlled trials, which are guiding physicians in the treatment decision-making process.

Despite significant improvements in the control of local disease, a significant number of patients ultimately die of recurrent/metastatic disease following radical surgery due to a lack of effective adjuvant treatments.

In addition, the characteristic chemoresistance of STS has compromised the therapeutic value of conventional antineoplastic agents in cases of unresectable advanced/metastatic disease.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Alveolar soft part sarcoma: a rare and enigmatic entity.

Alveolar soft part sarcoma is a rare malignant tumor with unusual clinical behavior.

Treatment of alveolar soft part sarcoma has been difficult to evaluate because of the small numbers of cases seen, but it seemed that although treatment of the primary tumor in alveolar soft part sarcoma often is successful, treatment of metastatic tumors is unsuccessful.

A review of outcome after treatment of primary and metastatic disease in the 15 patients in our database with alveolar soft part sarcoma was done in order to evaluate this issue.

Nine of 15 patients presented with metastatic disease and one further patient developed metastases.

Adjuvant chemotherapy was administered to one patient with localized disease and to six patients with metastatic disease.

Treatment of metastatic tumors involved chemotherapy in seven patients, metastectomy in three patients, and radiation in two patients.

All instances of the metastatic disease either recurred or progressed.

Overall survival was 75% at 5 years and 40% at 10 years with a mean survival of 6.5 years, despite the high number of patients with metastatic disease.

Current treatment results in good local control of primary tumors, but poor control of metastatic tumors.

New approaches to treatment of metastatic alveolar soft part sarcoma must be investigated and applied.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts.

Clear cell sarcoma is a high-grade sarcoma with morphological features resembling those of malignant melanoma.

An osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts is very rare.

Herein, we report an unusual stomach tumor with microscopic and immunohistochemical characteristics of an osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcomas of soft parts.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECTIVE: The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy.

(1) despite the globally good prognosis of grossly-resected cases, patients with G3 and large-size have a high-risk of metastatic spread, and (2) MFS appears to be better in patients who had chemotherapy.

Based in part on these results, and in accordance with recent suggestions coming from the literature on adult sarcomas, the EpSSG NRSTS protocol will recommend adjuvant chemotherapy in high-risk surgically-resected patients.

METHODS: Eligible patients were those who had histologic evidence of locally advanced or metastatic STS and who had received 1 prior treatment regimen with an anthracycline-based chemotherapy for metastatic disease.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies.

Differently from adult oncologists that considered synovial sarcoma (SS) a tumor with uncertain chemosensitiveness, since two decades pediatric oncologists in Europe assumed that chemotherapy played an important role in SS treatment, so most pediatric patients were included in rhabdomyosarcoma protocols, receiving adjuvant chemotherapy regardless of risk factors.

No metastatic relapses occurred in Group I < or = 5 cm patients, while the outcome was poor for T2B patients (EFS 41%) due to a high rate of metastatic relapse.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Partial hand preservation for large soft tissue sarcomas of the hand.

In patients with large soft tissue sarcomas of the hand, partial hand preservation is extremely challenging for surgeons attempting a complete resection of the tumor with negative resection margins.

We conducted this review to examine the oncologic outcome, including local recurrence rate and patient overall survival, and functional outcome after resections for large soft tissue sarcomas with partial hand preservation.

METHODS: We performed a retrospective review of all patients with soft tissue sarcomas of the hand treated at our institution from 1995 to 2007.

Two patients had myxofibrosarcoma, 2 patients had synovial sarcoma, 2 patients had malignant fibrous histiocytoma, 1 patient had a malignant peripheral nerve sheath tumor, and 1 patient had a liposarcoma.

Two patients had American Joint Committee on Cancer stage 1b tumors, and 6 patients had American Joint Committee on Cancer stage 3 tumors.

RESULTS: Of the 8 patients, 1 died of distant metastatic disease, 1 developed local tumor recurrence and is alive with locally recurrent disease, and the other 6 patients are completely disease-free.

CONCLUSIONS: Partial hand preservation is possible in selected patients with large soft tissue sarcomas of the hand, obtaining low local recurrence rates, good overall survival, and good functional outcome.

PATIENTS AND METHODS: Patients with metastatic or unresectable STS were randomly assigned to treatment with one of two ABT-510 dose schedules (20 mg once a day [20 mg], n = 42; or 100 mg twice a day [200 mg], n = 46), which were self-administered subcutaneously in 28-day treatment periods.